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Clinical Research
The Natural and Unnatural History of Congenital Aortic Arch
Abnormalities Evaluated in an Adult Survival Cohort
Joyce E. Lodeweges, MD,
a
Frederik G. Dikkers, MD, PhD,
b
Barbara J.M. Mulder, MD, PhD,
c
Jolien W. Roos-Hesselink, MD, PhD,
d
Hubert W. Vliegen, MD, PhD,
e
Arie P.J. van Dijk, MD, PhD,
f
Gertjan T. Sieswerda, MD, PhD,
g
Thelma C. Konings, MD,
h
Rolf M.F. Berger, MD, PhD,
i
Dirk-Jan Slebos, MD, PhD,
j
Tjark Ebels, MD, PhD,
k,l
and
Joost P. van Melle, MD, PhD
a
a
Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands;
b
Department of Otorhinolaryngology, Academic
Medical Center, Amsterdam, The Netherlands;
c
Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands;
d
Department of Cardiology, Erasmus
University Medical Center, Rotterdam, The Netherlands;
e
Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands;
f
Department of
Cardiology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands;
g
Department of Cardiology, University Medical Center Utrecht, Utrecht, The
Netherlands;
h
Department of Cardiology, Free University Medical Center, Amsterdam, The Netherlands;
i
Department of Pediatric Cardiology, Beatrix Children’s Hospital,
University Medical Center Groningen, University of Groningen, Groningen, The Netherlands;
j
Department of Pulmonary Diseases, University Medical Center Groningen,
University of Groningen, Groningen, The Netherlands;
k
Department of Cardiothoracic Surgery, University Medical Center Groningen, University of Groningen,
Groningen, The Netherlands;
l
Department of Cardio-Thoracic Surgery, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands
See editorial by Guron and Oechslin, pages 373e375 of this issue.
ABSTRACT
Background: This study describes the different types of congenital
vascular rings according to their anatomy, symptoms, and age at
clinical onset and reports the surgical outcomes.
Methods: A retrospective observational database study was conduct-
ed, reviewing the medical charts of 69 adult survivors with a history of
a vascular ring, identified from the Dutch Congenital Cor vitia
database.
Results: Median age at presentation was 8.5 years (0-53.0 years).
Thirty patients (43.5%) had a “left aortic arch with aberrant right
subclavian artery,”21 patients (30.4%) a “double aortic arch,”and 16
patients (23.2%) a “right aortic arch with aberrant left subclavian
artery.”The main symptomatology at presentation comprised respi-
ratory symptoms (82.9%). Almost three-quarters of patients were also
diagnosed with asthma/bronchial hyperreactivity. Patients with a
R
ESUM
E
Contexte : Cette
etude d
ecrit les diff
erents types d’anneaux vascu-
laires cong
enitaux selon leur anatomie, leurs symptômes et l’âge à
leur apparition clinique ainsi que les issues de l’intervention
chirurgicale.
M
ethodologie : Une
etude r
etrospective observationnelle a
et
e
r
ealis
ee à partir d’une base de donn
ees, dans laquelle ont
et
e analys
es
les dossiers m
edicaux de 69 adultes survivants ayant des ant
ec
edents
d’anneaux vasculaires rep
er
es dans la base de donn
ees des anomalies
cong
enitales CONgenital COR vitia des Pays-Bas.
R
esultats : L’âge m
edian à la survenue
etait de 8,5 ans (0-53,0 ans).
Trente patients (43,5 %) pr
esentaient un «arc aortique gauche avec
artère sous-clavière droite aberrante », 21 patients (30,4 %) avaient un
«double arc aortique »et 16 patients (23,2 %), un «arc aortique
droit avec artère sous-clavière gauche aberrante ». Les problèmes
The term “vascular ring”indicates a rare congenital vascular
anomaly.
1
It represents less than 1% of all congenital heart
defects,
2,3
due to an abnormal development of the branches of
the aortic arch. This causes vascular encirclement of the tra-
chea and/or esophagus, leading to variable degrees of
compression and giving rise to a wide range of respiratory and
gastrointestinal symptoms that vary in severity and age at
presentation.
3,4
Anatomically, different types of vascular rings
exist, which may be divided into 2 groups (complete and
incomplete rings).
5,6
Because of the low incidence of vascular
rings, their anatomical heterogeneity, and their nonspecific
symptomatology, this pathology is often hardly recognized,
leading to mis- or undiagnosed cases having lifelong com-
plaints.
7,8
The aim of this study was to describe the different
types of vascular rings, in a cohort of 69 patients, according to
Canadian Journal of Cardiology 35 (2019) 438e445
Received for publication June 23, 2018. Accepted December 4, 2018.
Corresponding author: Dr Joost P. van Melle, Department of Cardiology,
Thorax Center, University Medical Center Groningen, University of
Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands.
Tel.: þ31-50-361-3544.
E-mail: j.p.van.melle@umcg.nl
See page 444 for disclosure information.
https://doi.org/10.1016/j.cjca.2018.12.004
0828-282X/Ó2018 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
their anatomical heterogeneity, presenting symptoms, and
differences in age at clinical onset, to raise clinical awareness of
this complex congenital anomaly. The secondary objective was
to report the (long-term) surgical outcomes.
Patients and Methods
Study population
A retrospective observational database study was per-
formed, using the computerized coding system of the Dutch
Congenital Cor vitia (CONCOR) registry for adult patients
with congenital heart disease. From November 2001, Dutch
patients 18 years or older with a congenital heart defect have
been recruited in secondary and tertiary medical centres and,
after giving informed consent, included in this database. This
is a survival cohort because this database includes only patients
with a congenital cardiac anomaly who survived (and who are
not lost to follow-up) until the age of 18 years or more. In this
database, clinical events are coded using the European Pedi-
atric Cardiac Code Short List coding scheme.
9
For this study,
a selection of the European Pediatric Cardiac Code Short List
coding scheme was made, and all patients coded with
“vascular ring,”“aortic branch abnormality,”“right aortic
arch,”“double aortic arch (DAA),”“aberrant origin right
subclavian artery,”and “pulmonary artery sling”as code were
selected. A list of 209 adult patients from 7 academic centres
in The Netherlands with a possible complete or incomplete
vascular ring was obtained.
Patient and clinical variables
The medical charts of the adult patients identified from the
CONCOR database were examined. To determine the true
existence of a vascular ring, the operative reports and imaging
studies were reviewed. If no vascular ring was present, the pa-
tient was excluded and no further data were collected. If a
vascular ring was present, the following patient and treatment
information were obtained: date of birth, sex, type of vascular
ring, comorbidities, associated (cardiac) anomalies, date of
clinical onset, (presenting) symptoms, date of diagnosis, diag-
nostic strategies and their results, date and type of surgery,
postoperative complications and outcomes, length of hospital
stay, need for reoperation, and length of follow-up. The type of
vascular ring was classified according to the categories by Backer
and Mavroudis,
6
based on the anatomical characteristics of the
vascular ring. Of note, according to this established nomen-
clature of vascular rings, those aortic anomalies that compress a
portion of the esophagus or trachea, without encircling it as a
complete ring, were also considered a vascular ring.
The date of clinical onset was defined as the patient-
reported date of the first occurrence of symptoms, most
likely related to the vascular ring. To evaluate the possible
differences between patients with an early (ie, below 18 years)
or late onset (ie, 18 years or older) of symptoms, patients were
divided into 2 subgroups. The postoperative outcome was
divided into 3 categories: (1) cured (complete relief of
symptoms), (2) improved (decrease in severity, duration, or
number of episodes), and (3) no improvement.
9
Statistical analysis
Data were analyzed using IBM SPSS Statistics version 22.0
for Windows (IBM Corp, Armonk, NY). Descriptive statistics
were applied to demonstrate all variables. Continuous data
were presented as means and standard deviations when nor-
mally distributed or medians and range when nonnormally
distributed and categorical data as frequencies and percent-
ages. Comparison of categorical variables was made using the
c
2
test (or Fisher’s exact test). Comparison of 2 paired cate-
gorical variables was made using the Wilcoxon signed-rank
test. Comparison of continuous variables was made using
the independent sample ttest, Mann-Whitney Utest, or
Kruskal-Wallis Htest, depending on the number of groups
double aortic arch had more symptoms than patients with a left aortic
arch with aberrant right subclavian artery and right aortic arch with
aberrant left subclavian artery (P<0.001), requiring surgery most
often (P<0.001). In patients with childhood onset of symptoms,
preoperative spirometry (ie, peak expiratory flows) was more often
abnormal as compared with adult patients (P¼0.007). Surgery was
performed in 42.0% of all patients at a median age of 17 years (0-63.0
years). Twenty-four (92.3%) of the operated patients showed
improvement or complete relief of symptoms shortly after surgery. Of
26 asymptomatic nonoperated patients, 3 patients (11.5%) eventually
developed symptoms.
Conclusions: The low incidence of vascular rings, their anatomic het-
erogeneity, and a wide range of common symptoms often lead to
misdiagnosis. Clinical awareness is warranted as a large subset of
patients could benefit from surgery, even at an adult age.
respiratoires
etaient les principaux symptômes initiaux (82,9 %). Près
des trois quarts des patients avaient
egalement reçu un diagnostic
d’asthme ou d’hyperr
eactivit
e bronchique. Les patients pr
esentant un
double arc aortique avaient plus de symptômes que ceux qui
pr
esentaient un arc aortique gauche avec artère sous-clavière droite
aberrante ou un arc aortique droit avec artère sous-clavière gauche
aberrante (p<0,001) et avaient plus souvent besoin d’une chirurgie
(p<0,001). Chez les patients dont les symptômes
etaient apparus
pendant l’enfance, la spirom
etrie pr
eop
eratoire (c.-à-d. la mesure du
d
ebit expiratoire de pointe)
etait plus souvent anormale que chez les
patients adultes (p¼0,007). Une intervention chirurgicale avait
et
e
pratiqu
ee chez 42,0 % de tous les patients à un âge m
edian de 17 ans
(0-63,0 ans). Une att
enuation ou une disparition complète des symp-
tômes a
et
e observ
ee chez vingt-quatre (92.3%) des patients op
er
es,
peu de temps après la chirurgie. Sur 26 patients asymptomatiques et
non op
er
es, 3 patients (11,5 %) ont fini par pr
esenter des symptômes.
Conclusions : La faible incidence des anneaux vasculaires, leur
h
et
erog
en
eit
e anatomique et la vaste gamme de leurs symptômes les
plus courants conduisent souvent à des erreurs de diagnostic. Une
meilleure sensibilisation clinique s’impose, car un important sous-
ensemble de patients pourrait tirer profitd’une intervention chi-
rurgicale, même à l’âge adulte.
Lodeweges et al. 439
History of Congenital Aortic Arch Abnormalities
and distribution of data. Associations between continuous and
ordinal variables were assessed using the Kendall Tau-b cor-
relation coefficient. All variables were visually checked for
normality using Q-Q plots and histograms. In case of doubt,
normality was tested using the Shapiro-Wilk test. A Pvalue of
<0.05 was considered statistically significant.
Results
Demographics
Of 209 patients, 140 patients were excluded, because they
did not meet the criteria of a vascular ring (eg, some appeared to
have a “right arch”only). Also, no patients with a pulmonary
sling could be identified. In total, 69 patients with a vascular
ring were included in this study. At the time of data extraction,
15,602 patients were included in the CONCOR database,
leading to a point prevalence of vascular rings in the adult
population with a congenital heart defect of 0.44%. The
demographic characteristics are depicted in Table 1. The
median age of diagnosis for all patients, including the asymp-
tomatic patients, was 11.0 years (range, 0-70.0 years).
Comorbidities were found in 23 patients (33.3%), with
asthma/bronchial hyperreactivity being the most frequently
diagnosed comorbid disease (73.9%) and being significantly
more common in patients with a DAA than in patients with
other types of vascular rings (52.9% vs 47.0%, P¼0.046).
Associated cardiac anomalies were found in 44 (63.8%)
patients.
Type of vascular rings
The distribution of vascular rings in the study population
and the associated age at clinical onset and the age at surgery
are shown in Table 2. Of the 69 patients with a vascular ring,
30 (43.5%) had a “left aortic arch with aberrant right sub-
clavian artery”(LAARSA) (Fig. 1), 21 (30.4%) a DAA
(Fig. 1), and 16 (23.2%) a “right aortic arch with aberrant left
subclavian artery”(RAALSA). One patient had an “innomi-
nate artery compression”and 1 patient a “right cervical aortic
arch.”A Kommerell’s diverticulum was reported in 8 (11.6%)
of the 69 patients.
Presenting symptoms
Of all patients, including patients with innominate artery
compression and right cervical aortic arch, 37 patients became
symptomatic (53.6%). The median age at clinical onset was 8.5
years (range, 0-53.0 years). Twelve patients (32.4%) had
symptoms present early after birth. There was no significant
difference in age at clinical onset between the different types of
vascular rings (P¼0.738, Table 2). The median age of diag-
nosis for the symptomatic patients was 16.0 years with a wide
range from 0 to 63.0 years. Three patients (8.1%) were diag-
nosed before symptoms started. Thirteen patients (35.1%) had
a delay in the diagnosis of 1 year or more, with a median delay of
11.0 months (range, 1.0-639.0 months). Patients with a DAA
were more often symptomatic than patients with a RAALSA
and LAARSA (85.7% vs 50.0% and 30.0%, respectively, P<
0.001). Respiratory symptoms were the most common type of
symptoms (n ¼29, 82.9%), with a significant predominance
for patients with a DAA (ie, 81.0% vs 37.5% for RAALSA and
20.0% for LAARSA, P<0.001). Dyspnea was the most
common respiratory symptom (n ¼25, 86.2%). A significant
difference between the different types of vascular rings was
found for dyspnea (P<0.001), recurrent respiratory tract in-
fections (P¼0.009), stridor (P¼0.001), chest pain
(P¼0.037), and cyanosis (P¼0.043), all of them
Table 1. Demographic characteristics of a cohort of 69 patients with a
vascular ring
Demographics Value
Sex
Female 41 (59.4)
Age at diagnosis (y) 11.0 (0-70.0)
Comorbidities (n ¼23)
Asthma/bronchial hyperreactivity 17 (73.9)
Atopy 5 (21.7)
Hypertension 7 (30.4)
Diabetes mellitus 1 (4.3)
Renal failure 1 (4.3)
Associated cardiac anomalies (n ¼44)
Ventricular septal defect 19 (43.2)
Coarctation of the aorta 13 (29.5)
Atrial septal defect 8 (18.1)
Tetralogy of Fallot 8 (18.1)
Transposition of the great arteries 4 (9.1)
Patent ductus arteriosus 3 (6.8)
Values are given as numbers (%) or medians (range).
Table 2. Distribution of vascular rings with ages at clinical onset (CO) and surgery
Type of vascular ring Patients Age at CO (y) Age at surgery (y)
Double aortic arch 21 (30.4) 8.0 (0-53.0) 10.0 (0-55.0)
Balanced arches 2 (9.5)
Left arch dominant 1 (4.8)
Right arch atretic e
Right arch patent 1 (100.0)
Right arch dominant 14 (66.7)
Left arch atretic 6 (42.9)
Left arch patent 5 (35.7)
Unknown 4 (19.0)
Right aortic arch, left ligament, aberrant left subclavian artery 16 (23.2) 3.0 (0-30.0) 11.0 (0-44.0)
Left aortic arch, aberrant right subclavian artery 30 (43.5) 23.0 (0-39.0) 23.0 (0-63.0)
Innominate artery compression 1 (1.4) 26.0 (e) 28.0 (e)
Right cervical aortic arch 1 (1.4) 8.0 e
Total 69 (100.0) 8.5 (0-53.0) 12.0 (0-63.0)
Values are given as numbers (%) or medians (range).
440 Canadian Journal of Cardiology
Volume 35 2019
predominating in patients with a DAA. Gastrointestinal
symptoms were found 13 times (35.1%), with dysphagia for
solid foods being the most common (n ¼9, 69.2%) (Table 3).
Characteristics according to the age of clinical onset
The 2 subgroups of symptomatic patients according to the
age of clinical onset (ie, childhood vs adulthood) consisted of
24 (64.9%) and 13 (35.1%) patients, respectively. No
significant difference in the types of vascular rings between the
adult and the paediatric group existed.
The number of respiratory or gastrointestinal symptoms did
not differ significantly between the paediatric and adult groups
(P¼0.643 and P¼0.472, respectively). Only chest pain was
significantly more common in the adult group (P¼0.042).
Preoperative spirometry was performed in 16 of 37
symptomatic patients (43.2%). Of them, 9 patients (56.3%)
had paediatric onset and 7 patients (43.7%) adult onset
1
2
3
4
5
6
7
8
9
10
11
Esophagus
Trachea
Left recurrent laryngeal nerve
Right subclavian artery
Right carotid artery
Left carotid artery
Left subclavian artery
Aortic arch
Ligamentum arteriosum
Pulmonary artery
Left aortic arch (atretic)
hcra citroa elbuoDyretra naivalcbus tnarrebA
10
4
6
8
911
7
5
3
2
1
10
4
6
8
9
7
5
3
2
1
Figure 1. The 2 most common vascular rings: a left aortic arch with aberrant right subclavian artery constituting an incomplete ring and a double
aortic arch. Of note, in the latter variant, the left arch is partially atretic, which cannot be visualized easily with current imaging modalities as the
atretic parts do not fill with contrast medium.
Table 3. Distribution of presenting symptoms according to the type of vascular ring
Symptoms DAA (n ¼21) RAALSA (n ¼16) LAARSA (n ¼30) Total Pvalue
Respiratory 17 (81.0) 6 (37.5) 6 (20.0) 29 (82.9) <0.001*
Dyspnea 17 (81.0) 5 (31.3) 3 (10.0) 25 (86.2) <0.001*
Recurrent RTI 7 (33.3) 2 (12.5) 1 (3.3) 10 (34.5) 0.009
Stridor 6 (28.6) ee6 (20.7) 0.001
Cough 4 (19.0) 3 (18.8) 1 (3.3) 8 (27.6) 0.088
Wheezing 4 (19.0) 1 (6.3) e5 (17.2) 0.092
Chest pain 3 (14.3) e3 (10.0) 6 (20.7) 0.037
Cyanosis 4 (19.0) 1 (6.3) e5 (17.2) 0.043
Dysphonia 1 (4.8) ee1 (3.4) 0.552
Gastrointestinal 3 (14.3) 4 (25.0) 6 (20.0) 13 (37.1) 0.622
Dysphagia for solid foods 3 (14.3) 2 (12.5) 4 (13.3) 9 (69.2) 0.470
Weight loss ee4 (13.3) 4 (30.8) 0.068
Emesis e1 (6.3) 2 (6.7) 3 (23.1) 0.353
Gastroesophageal reflux 2 (9.5) 1 (6.3) e3 (23.1) 0.112
Nausea e1 (6.3) e1 (7.7) 0.096
Total 18 (85.7) 8 (50.0) 9 (30.0) 35 (100.0) <0.001
Values are given as numbers (%). Pvalues refer to Fisher’s exact test. Because “innominate artery compression”and “right cervical aortic arch”represented only 1
patient per group, these types of vascular rings were excluded from statistical analysis in this table. The bold Pvalues are significant.
DAA, double aortic arch; LAARSA, left aortic arch, aberrant right subclavian artery; RAALSA, right aortic arch, aberrant left subclavian artery; RTI, respiratory
tract infection.
*
c
2
test.
Lodeweges et al. 441
History of Congenital Aortic Arch Abnormalities
(Table 4). Preoperative forced vital capacity (FVC), forced
expiratory volume in 1 second, and forced expiratory flow
after 75% of FVC expired values appeared to be normal in
both groups, with mean percentages of predicted above 80%.
Preoperative peak expiratory flow (PEF), forced expiratory
flow after 25% of FVC expired, and forced expiratory flow
after 50% of FVC expired values were found to be signifi-
cantly lower and below normal in patients with paediatric
onset, with mean percentages of predicted under 80%
(independent sample ttest, P¼0.007, P¼0.038, and
P¼0.026, respectively). A plateau in the expiratory
flow-volume curve was found in 55.6% and 71.4% of patients
with paediatric and adult onset, respectively. Figure 2 shows a
flow-volume curve of a representative patient from the study
population.
Surgery
When looking at surgery details (Table 5), 29 of 69
patients underwent surgery (42%) at a median age of 17 years
(range, 0-63 years), with significant predominance for patients
with a DAA (16 of 21 patients with a DAA were operated)
compared with patients with a RAALSA and LAARSA
(76.2% vs 43.8% and 16.7%, P<0.001). Seven of 8 patients
with a Kommerell’s diverticulum were operated (87.5%).
There was no significant difference in age at surgery between
the different types of vascular rings (Kruskal-Wallis Htest,
P¼0.135, Table 2). Symptoms were present in 26 (89.6%)
of these 29 operated patients. Three asymptomatic patients
underwent surgery because it was expected that they would
develop symptoms. Of the 40 patients who did not undergo
surgery, 29 (72.5%) were asymptomatic. The other 11
(symptomatic) patients (27.5%) did have mild symptoms not
requiring surgery or were inoperable because of associated
cardiac anomalies. The most often performed approach of
surgery to divide the arch was a left thoracotomy (n ¼16,
55.2%). Complications were registered in 7 patients (24.1%)
and included transient vocal cord paresis (n ¼4), pneumo-
thorax (n ¼2), chylothorax (n ¼1), and Horner syndrome
(n ¼1). A reoperation was required in 5 patients (17.2%) and
included aortopexy, tracheopexy, tracheoplasty, tracheal
dilatation with a patch, and removal of fibrotic tissue. No
patients died during or shortly after surgery.
Postoperative outcomes and follow-up
The median length of hospital stay was 9.0 days (range,
4.0-130.0 days). Figure 3 shows the differences in post-
operative outcomes at the first (median 1 month after surgery)
and last follow-up (median 5 years after surgery) for the
symptomatic patients (n ¼26). Of all symptomatic, operated
patients, 92.3% showed improvement or complete relief of
symptoms at the first follow-up. The outcome score did not
Table 4. Preoperative spirometry results according to the age of clinical onset (CO)
Spirometry (n ¼16) Patients CO <18 (n ¼9) Value Patients CO 18 (n ¼7) Value Pvalue
FVC (L) 6 4.17 1.17 6 4.02 1.13 0.821
FVC% of predicted 6 101.8 17.8 6 103.3 21.5 0.898
FEV
1
(L) 7 2.82 0.84 7 2.87 0.96 0.931
FEV
1
% of predicted 8 81.9 19.9 7 96.0 20.1 0.196
PEF (L/s) 5 3.81 0.83 5 6.98 1.80 0.007
PEF% of predicted 5 58.4 4.3 5 90.2 19.5 0.007
FEF
25
(L/s) 4 3.35 0.83 3 5.42 1.00 0.030
FEF
25
% of predicted 4 57.3 8.2 2 88.0 18.4 0.038
FEF
50
(L/s) 4 2.64 0.49 5 4.22 0.60 0.004
FEF
50
% of predicted 4 65.3 3.3 5 94.4 19.3 0.026
FEF
75
(L/s) 4 1.81 0.55 3 1.63 0.59 0.695
FEF
75
% of predicted 4 84.3 17.0 2 83.0 29.7 0.948
Plateau expiratory flow-volume curve 5 5 0.462*
Plateau inspiratory flow-volume curve 2 e0.303*
Values are given as numbers. Pvalues in the table refer to the independent sample ttest. The bold Pvalues are significant.
FEF
25
, forced expiratory flow at 25% of the forced vital capacity; FEF
50
, forced expiratory flow at 50% of the forced vital capacity; FEF
75
, forced expiratory flow
at 75% of the forced vital capacity; FEV
1,
forced expiratory volume in 1 s; FVC, forced vital capacity; PEF, peak expiratory flow.
* Fisher’s exact test.
Figure 2. Spirometry performed in a representative patient from the
study population with a double aortic arch. The flow-volume curve
shows a sudden drop in the expiratory flow after the forced expiratory
flow (PEF), followed by a plateau phase, consistent with variable
intrathoracic obstruction during forced expiration.
442 Canadian Journal of Cardiology
Volume 35 2019
significantly change between the first and last time of follow-
up (Wilcoxon signed-rank test, P¼0.109, with a median
outcome score of “2”[improvement] both at the first and last
follow-up). There was no significant correlation between the
outcome at the first or last postoperative follow-up and the age
at surgery (P¼0.065 and P¼0.110, respectively).
The median length of follow-up of all patients was 18 years
(range, 0-63.0 years). Four patients (10.0%), who were not
operated, died during the follow-up at35, 36, 38, and 67 years of
age, due to congenital heart disease. Of 10 nonoperated, symp-
tomatic patients, 6 patients (60.0%) had spontaneous relief of
symptoms. Of 26 asymptomatic patients who did not undergo
surgery, 3 patients (11.5%) eventually developed symptoms.
Discussion
Vascular rings are congenital anomalies characterized by an
abnormal development of the aortic arch. This causes com-
plete or partial encirclement of the trachea and/or esophagus
and results in a wide range of symptoms, also seen in other
more common diseases, which often complicate the diag-
nosis.
1,2,4
This retrospective observational database study is, to
our knowledge, the largest in the world, reporting character-
istics of 69 adult patients with a history of vascular rings.
Vascular rings
In this study, a point prevalence of vascular rings in the
adult population with a congenital heart defect of 0.44% was
found. This is in line with the percentages of less than 1%
stated in previous reports.
2,3
A complete vascular ring was
present in more than half of the patients and comprised 21
patients (30.4%) with a DAA, with the right arch being
dominant in most cases (66.7%), and 16 patients (23.2%)
with a RAALSA. The vast majority of patients with an
incomplete ring constituted of patients with a LAARSA.
These findings are in line with the previous literature,
10-12
reporting DAA as the most common type of a complete
vascular ring, with the right arch being dominant most of the
times, and LAARSA as the most common aortic arch anomaly
overall. However, several reports described a much lower
number of patients with a LAARSA.
13-15
This may be
explained by the predominantly asymptomatic course of this
type of vascular ring, which can let this ring undiagnosed or
only found on imaging studies by coincidence.
10,16
In our
study, LAARSA was found coincidentally on imaging in 70%
of all patients with a LAARSA.
Vascular rings are frequently seen in association with other
congenital cardiac anomalies (63.8% in this series, compared
with 12% to 63% in previous series
12,14,17,18
), which may
dominate the clinical presentation and complicate the diag-
nosis of a vascular ring.
Presenting symptoms
Symptoms were present in approximately half of the
patients, with predominance of patients with a DAA. In one-
third of the symptomatic patients, symptoms presented at
birth. In line with earlier series,
13,17,19
respiratory symptoms
were the most common type of symptoms in this study.
Dysphagia for solid foods was the most frequent gastrointes-
tinal symptom and, in line with previous data,
20
most
commonly presented in patients with a LAARSA.
In approximately one-third of the patients, comorbidities
were found and included mostly asthma/bronchial hyperre-
activity, especially in patients with a DAA. However, it is not
clear from this data set whether these cases of asthma/bron-
chial hyperreactivity were true comorbidities or mis-
interpretations of symptoms of a vascular ring. For example,
one of the patients in our study used bronchodilators for over
10 years, which could be terminated after surgery for her
vascular ring. Previous reports
7,8
emphasize this risk of mis-
diagnosing DAA as asthma, which delayed the true diagnosis,
delineating the importance of considering vascular rings in the
differential diagnosis of patients with respiratory symptoms.
Characteristics according to the age of clinical onset
Previous literature reported respiratory symptoms to be
more frequent in infants and children and gastrointestinal
Table 5. Treatment details according to the type of vascular ring
Variables DAA (n ¼21) RAALSA (n ¼16) LAARSA (n ¼30) INN (n ¼1) RCAA (n ¼1) Total Pvalue
Surgery performed 16 (76.2) 7 (43.8) 5 (16.7) 1 (100.0) e29 (42.0) <0.001
Age at surgery (y) 10 (0-55) 11 (0-44) 23 (0-63) 28 (e)e17 (0-63) e
Complications 3 (18.8) 3 (42.9) 1 (20.0) ee7 (24.1) 0.621
Reoperation required 3 (18.8) 1 (14.3) e1 (100.0) e5 (17.2) 0.221
Values are given as numbers (%) or as medians (range). Pvalues in the table refer to Fisher’s exact test. The bold Pvalues are significant.
DAA, double aortic arch; INN, innominate artery compression; LAARSA, left aortic arch, aberrant right subclavian artery; RAALSA, right aortic arch, aberrant
left subclavian artery; RCAA, right cervical aortic arch.
Figure 3. Postoperative outcomes at the first and last time of
follow-up.
Lodeweges et al. 443
History of Congenital Aortic Arch Abnormalities
symptoms to be more common in adults.
19
In this study, no
significant difference in the number of respiratory and
gastrointestinal symptoms between the paediatric and adult
groups existed.
More than half of patients with a DAA presented in
infancy or childhood. This is consistent with the previous
literature,
21
reporting DAA to be less common in adults,
because of its tendency to cause severe respiratory symptoms,
leading to early diagnosis and correction. In LAARSA,
symptoms were present in only one-third of patients with a
median age at the onset of symptoms of 23.0 years. These
findings agree with data previously reported,
10,16
that is, that
LAARSA usually produces no symptoms and is commonly
discovered coincidentally on imaging. However, when the
aberrant subclavian artery becomes tortuous and dilated in the
elderly, late onset of symptoms such as dysphagia due to
esophageal compression (“dysphagia lusoria”) may emerge.
20
Remarkably, preoperative spirometry was obtained in less
than half of symptomatic patients, suggesting that this test is
underused for these (respiratory) symptoms. Spirometry
showed a plateau in the expiratory flow-volume curve in more
than three-quarters of patients in the paediatric group and a
decreased value for PEF, forced expiratory flow after 25% of
FVC expired, and forced expiratory flow after 50% of FVC
expired, indicating a variable intrathoracic obstruction and
thereby suggesting a vascular ring.
22,23
In the adult group, all
preoperative values were normal. This may indicate a more
severe intrathoracic obstruction due to the vascular ring in the
paediatric group, leading to an earlier onset of symptoms.
Rather than FEV
1
or FVC, the spirometric values most often
examined by clinicians, tracheal obstruction is better recog-
nized looking at PEF in spirometry. Although not specificor
sensitive, the presence of a variable intrathoracic obstruction
pattern in spirometry should raise suspicion for a vascular ring
and prompt further investigation.
22
Surgery, postoperative outcomes, and follow-up
Surgery was performed in 42.0% of patients at a median
age of 17 years, with most surgical repairs in patients with
DAA. Those who did not undergo surgery were asymptom-
atic, inoperable, or had mild symptoms not requiring surgery.
Only 3 asymptomatic patients who were not operated even-
tually developed symptoms, supporting a “wait and see”policy
for patients with no symptoms.
10,24
In agreement with earlier
literature,
10,16,24
a left thoracotomy was the most performed
type of surgery in most types of vascular rings. As described in
earlier reports,
13,18,24
surgical correction could be achieved
without intra- and postoperative mortalities. According to the
literature,
13,14
most patients showed (some) improvement or
complete relief of symptoms after surgery at the first
follow-up. Relief of symptoms may be hampered immediately
because of associated tracheomalacia.
16
At the last post-
operative follow-up, almost one-third of patients was consid-
ered cured and only 1 patient had still no improvement in
symptoms. In some patients, surgery may not resolve all
airway symptoms when the extrinsic tracheal compression is
associated with an intrinsic weakness of the cartilaginous ring
(tracheomalacia). It is an important prognostic factor in the
management of patients with a vascular ring because it
determines postoperative outcome.
25
In contrast to earlier
reports describing a relation between postoperative outcome
and the age of surgery,
10,15
no significant correlation was
found in the current study. This may be attributed to the
small size of the study population.
Limitations
The main limitation of this study is its retrospective design
and the fact that the CONCOR database is a survival cohort,
only including patients with a congenital cardiac anomaly who
survived and followed up until the age of 18 years or more.
Patients with a history of a vascular ring who were lost to
follow-up before the age of 18 years are therefore missed.
Secondly, the study population has a large heterogeneity with
different physicians and different surgeons from 7 medical
centres.
Conclusions
Vascular rings are relatively rare, congenital vascular
anomalies. The most common vascular ring is a LAARSA.
This is followed by DAA, in which symptoms are present
most frequently and most severely, requiring surgery most
often. The low incidence of vascular rings, their anatomic
heterogeneity, and the wide range of common respiratory and
gastrointestinal symptoms often lead to misdiagnosis as
asthma/bronchial hyperreactivity or gastroesophageal reflux
disease. In patients with suspected asthma who do not
respond to guideline therapy, or in whom the diagnosis is
doubtful, tracheal pathology (such as subglottic stenosis or a
vascular ring) should therefore be considered. In addition,
vascular rings are frequently associated with congenital cardiac
anomalies, which may dominate the clinical picture and may
complicate the diagnosis of a vascular ring as well. Although
not specific, additional lung function assessment may reveal
signs of a variable intrathoracic obstruction, raising the sus-
picion of a vascular ring. Surgical repair is required in patients
with symptoms and may lead to improvement in most
patients, even at an adult age. Therefore, clinical awareness for
this condition is warranted.
Acknowledgement
We thank Amanda Gautier, Scientific Illustrator of
Figure 1.
Disclosures
The authors declare that they have no relevant conflicts of
interest to disclose.
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